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Cutaneous T‐cell lymphoma: 2017 update on diagnosis, risk‐stratification, and management
Author(s) -
Wilcox Ryan A.
Publication year - 2017
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.24876
Subject(s) - medicine , mycosis fungoides , lymphoma , oncology , cutaneous t cell lymphoma , disease , stage (stratigraphy) , chemotherapy , t cell lymphoma , lymphoproliferative disorders , dermatology , paleontology , biology
Disease overview Cutaneous T‐cell lymphomas are a heterogenous group of T‐cell lymphoproliferative disorders involving the skin, the majority of which may be classified as Mycosis Fungoides (MF) or Sézary Syndrome (SS). Diagnosis The diagnosis of MF or SS requires the integration of clinical and histopathologic data. Risk‐adapted therapy TNMB (tumor, node, metastasis, blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a “risk‐adapted,” multi‐disciplinary approach to treatment. For patients with disease limited to the skin, expectant management or skin‐directed therapies is preferred, as both disease‐specific and overall survival for these patients is favorable. In contrast, patients with advanced‐stage disease with significant nodal, visceral or blood involvement are generally approached with biologic‐response modifiers or histone deacetylase inhibitors prior to escalating therapy to include systemic, single‐agent chemotherapy. In highly‐selected patients, allogeneic stem‐cell transplantation may be considered, as this may be curative in some patients.